Radiology Case Reports xxx (2015) 1e4

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Case Report Pseudoangiomatous stromal hyperplasia of the : a case report of a 12-year-old girl

Eman Almohawes MDa,*, Nuha Khoumais MDb, Maria Arafah MDc a Department of Radiology, Dammam Medical Complex, Dammam, Saudi Arabia b Department of Radiology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia c Department of Pathology, College of Medicine, King Saud University and King Khalid University Hospital, Riyadh, Saudi Arabia article info abstract

Article history: Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a benign lesion, charac- Received 30 May 2015 terized by a dense proliferation of stromal mesenchymal cells of myofibroblastic origin Accepted 11 June 2015 forming empty, slit-like channels. We report PASH in a 12-year-old girl with a huge rapidly Available online xxx enlarged right breast. Biopsy of the mass showed histopathologic features characteristic of PASH. Immunohistochemical studies revealed diffuse positive membranous immunore- Key words: activity to CD34. Although it is a benign lesion, lumpectomy was performed to minimize Pseudoangiomatous stromal hyper- the damage from developing breast tissue. plasia Copyright © 2015, the Authors. Published by Elsevier Inc. under copyright license from the University of Washington. This is an open access article under the CC BY license (http:// Ultrasound creativecommons.org/licenses/by/4.0/). Angiosarcoma

Introduction Case report

Pseudoangiomatous stromal hyperplasia (PASH) is an un- A 12-year-old girl, complaining of an enlargement of her right usual benign breast condition that was first described in breast, presented for diagnostic work-up. She mentioned that 1986 [1]. The reported age range of patients is 14-67 years, 2 months ago she had palpated a small nodule near the although the vast majority of PASH patients present in , which thereafter rapidly increased in size. There was their late thirties and forties [2]. It is histologically defined no nipple discharge. She had an unremarkable personal and as a complex network of slit-like spaces lined by family history, with reported menarche at 11 years and a endothelial-like spindle cells against a background of normal menstrual cycle. stromal hyperplasia [3]. On histologic analysis, PASH can be Physical examination revealed an overall enlarged right mistaken for a low-grade angiosarcoma and phyllodes breast replaced with a huge lump occupying the whole breast, tumor, but it is a benign condition. Correct identification of with diffuse tenderness and firmness on palpation. There was PASH of the breast has important treatment implications no evidence of thickening of the breast skin or nipple retrac- for patients [3-5]. tion. (Fig. 1).

* Corresponding author. Eman Almohawes. E-mail address: [email protected] (E. Almohawes). http://dx.doi.org/10.1016/j.radcr.2015.06.007 1930-0433/Copyright © 2015, the Authors. Published by Elsevier Inc. under copyright license from the University of Washington. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). 2 Radiology Case Reports xxx (2015) 1e4

mass measuring 17.3 cm at maximum diameter with cystic channels noted within the mass. The ipsilateral axilla showed benign-looking lymph nodes (Fig. 2). The differential diagnosis at this point included low-grade angiosarcoma, PASH, and phyllodes. Therefore, sampling was advised by the Breast Imaging-Reporting and Data System (BIRADS 4). Under ultrasonography guidance, core needle biopsy was performed, which showed the presence of spindle cells in the fibrous tissue in a pseudoangiomatous pattern, fitting the diagnosis of PASH. A complex pattern of anastomosing slit-like spaces in a dense collagenous stroma was seen. These spaces are lined with myofibroblasts rather than endothelial cells and do not contain red blood cells. This was the same finding on the final histopathology after excision (Fig. 3-5). Immunohistochemical studies revealed diffuse positive membranous immunoreactivity to CD34. In addition, negative staining was observed for CD31, a cellular adhesion marker highly restricted to endothelial neoplasms, and for desmin, a marker for cytoplasmic intermediate filaments found in the smooth muscle. The results of this panel of studies confirmed Fig 1 e Image showing the asymmetry and the large mass the characteristic immunophenotype of PASH while helping in the right breast on presentation. exclude lesions of vascular endothelial and smooth muscle origin such as angiosarcoma. The patient was finally operated; lumpectomy with reduction mammoplasty was performed. The patient had The patient was initially examined with breast ultraso- excellent cosmetic results postoperatively. The patient has nography, with a high frequency (8-10 MHz) linear array head, been followed up for 6 months now without evidence of which showed a huge right breast heterogeneous hypoechoic recurrence (Fig. 6).

Fig 2 e Ultrasonography showed a huge right breast with well-defined heterogeneous hypoechoic mass measuring 17.3 cm at maximum diameter, with cystic channels noted within the mass and no significant vascularity detected on Doppler imaging. Radiology Case Reports xxx (2015) 1e4 3

Fig 3 e Pseudoangiomatous stromal hyperplasia. A Fig 5 e Pseudoangiomatous stromal hyperplasia. A photomicrograph showing a cellular stromal lesion photomicrograph showing the myofibroblast cells lining between the bland breast ducts (hematoxylin and eosin, the vascular-like (pseudoangiomatous) spaces. The magnification £100). myofibroblast cells are spindle attenuated with bland spindle nuclei (hematoxylin and eosin, magnification £400).

Angiosarcoma often shows a more aggressive infiltrative Discussion pattern into surrounding fibroadipose breast tissue and is highly vascular, lined by endothelial cells. It does not show a PASH is a benign proliferation of the mammary stromal tissue. collagenous stroma. Angiosarcoma displays positive immuno- On histologic analysis, it shows complex interanastomosing reactivity for CD31 and, although less frequently and with less slit-like spaces which appear to be lined by spindle cells in the sensitivity, for the factor VIIIerelated or von Willebrand factor breast parenchyma [1]. antigens [7]. We present a case of a huge, rapidly growing PASH in a PASH is associated with several benign entities including 12-year-old patient, which is not frequently seen at this age proliferative and nonproliferative fibrocystic changes, such group; to our knowledge, the youngest female patient reported as fibroadenomas, , normal breast tissue, and in literature with PASH is a 10-year-old girl [6].This tumor sclerosing lobular hyperplasias. The etiology and pathogen- mimicked a low-grade angiosarcoma on ultrasonography; true esis remain unknown. cut biopsy revealed PASH, and surgical excision was performed. Hormonal factors are thought to play a role in the etiology It is important to differentiate the lesion from angio- of PASH [8]. sarcoma. It can be confused histologically with PASH. Microscopically, PASH consists of a network of slit-like spaces lined by myofibroblasts that resemble vascular spaces. On immunohistochemistry, PASH is positive for CD34 and vimentin and negative for factor VIIIerelated antigen and cytokeratins [9,10]. On radiologic examination, there are no specific or diag- nostic features. Most of these tumors mimic fibroadenomas or hamartomas and angiosarcomas, and larger tumors can be mistaken for phyllodes [9,11,12]. Management with wide local excision is the treatment of choice for PASH owing to its uncertain natural history [13]. The recurrence rates of PASH after excision are reported to range from 15% to 22% although longer follow-up studies are needed to evaluate the recurrence rate. The reason for recurrence could be attributed to the growth of a residual mass after incomplete excision [14,15].

Fig 4 e Pseudoangiomatous stromal hyperplasia. A Conclusion photomicrograph showing the anastomosing vascular-like (pseudoangiomatous) channels expanding the interlobular PASH is a benign entity that is regarded as a myofibroblastic stroma (hematoxylin and eosin, magnification £100). proliferation of the breast. 4 Radiology Case Reports xxx (2015) 1e4

Fig 6 e Postsurgical excision specimen. Postoperative pictures show good cosmetic results with slight asymmetry between both sides.

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